Imaging findings in faciobrachial dystonic seizures associated with LGI-1 antibodies

Achados de imagem em crises distônicas braquiofaciais associadas à anticorpos anti-LGI-1

A 76-year-old woman presented with a three-month history of faciobrachial dystonic seizures associated with cognitive impairment and hyponatremia. Cerebrospinal fluid analysis was unremarkable. A whole-body CT scan demonstrated no malignancies. Brain MRI revealed involvement of the mesial right temporal lobe, right striatum and globus pallidus and bilateral supratentorial white matter (Figures A, B, C). Leucine-rich glioma inactivated protein 1 (LGI-1) antibodies were detected in the serum. The patient received immunosuppressive treatment, with a partial response. LGI-1 encephalitis is an immune-mediated encephalitis characterized by cognitive impairment associated with faciobrachial dystonic seizures and hyponatremia¹. Neuroimaging findings include mesial temporal lobes, basal ganglia and supratentorial white matter abnormalites²,³.

Figure
Fluid-attenuated inversion recovery (FLAIR) images (A-C) disclosed increased signal in the right caudate and putamen (arrow in A); right hippocampus and amygdala (arrow in B); and slight blurring of the bilateral supratentorial white matter (arrows in C). A small remote vascular injury in the right occipital pole was also noticed, but considered unrelated to the case (images A-B).

References

• ¹
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  » https://doi.org/10.1212/WNL.0b013e318224afde
• ²
  Plantone D, Renna R, Grossi D, Plantone F, Iorio R. Teaching neuroimages: basal ganglial involvement in facio-brachial dystonic seizures associated with LGI1 antibodies. Neurology. 2013;80(17):e183-184. doi:10.1212/WNL.0b013e31828f17fa
  » https://doi.org/10.1212/WNL.0b013e31828f17fa
• ³
  Urbach H, Rauer S, Mader I, Paus S, Wagner J, Malter MP et al. Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis. Neuroradiology. 2015;57(12):1203-9. doi:10.1007/s00234-015-1581-x
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Publication Dates

• Publication in this collection
  Nov 2016

History

• Received
  19 July 2016
• Accepted
  10 Aug 2016